Chronic mucocutaneous candidiasis is a form of candidiasis (moniliasis) that usually develops during the first year of life but occasionally occurs as late as the 20s. Affecting males and females, it’s characterized by repeated infection with Candida albicans that may result from an inherited defect in cell-mediated (T-cell) immunity. (Humoral [B-cell] immunity remains intact and provides a normal antibody response to C. albicans.) In some patients, an autoimmune response affecting the endocrine system may induce various endocrinopathies.
Despite chronic candidiasis, these patients seldom die of systemic infection. Instead, they usually die of hepatic or endocrine failure. The prognosis for chronic mucocutaneous candidiasis depends on the severity of the associated endocrinopathy. Patients with associated endocrinopathy seldom live beyond their 30s.
No characteristic immunologic defects have been identified in this infection, but many patients have a diminished response to various antigens or to Candida alone. In some patients, anergy may result from deficient migration inhibition factor, a mediator normally produced by lymphocytes.
Candida species infections are the most common causes of fungal infections among patients who are immunocompromised. About 3 of every 4 females have at least one bout of vulvovaginal candidiasis during their lifetimes. In individuals who are HIV-positive, more than 90% experience oropharyngeal candidiasis and 10% have at least one episode of esophageal candidiasis.
Chronic candidal infections can affect the skin, mucous membranes, nails, and vagina, usually causing large, circular lesions. These infections seldom produce systemic symptoms but in late stages may be associated with recurrent respiratory tract infections. Other associated conditions include severe viral infections that may precede the onset of endocrinopathy and, sometimes, hepatitis. Involvement of the mouth, nose, and palate may cause speech and eating difficulties.
Symptoms of endocrinopathy are peculiar to the organ involved. Tetany and hypocalcemia are most common and are associated with hypoparathyroidism. Addison’s disease, hypothyroidism, diabetes, and pernicious anemia are also connected with chronic mucocutaneous candidiasis. Psychiatric disorders are likely because of disfigurement and multiple endocrine aberrations.
Laboratory findings usually show a normal circulating T-cell count, although it may be decreased. Skin tests don’t usually show delayed hypersensitivity to Candida, even during the infectious stage. Migration inhibiting factor that indicates the presence of activated T cells may not respond to Candida.
Nonimmunologic abnormalities resulting from endocrinopathy may include hypocalcemia, abnormal hepatic function studies, hyperglycemia, iron deficiency, and abnormal vitamin B12 absorption (pernicious anemia). Diagnosis must rule out other immunodeficiency disorders associated with chronic Candida infection, especially DiGeorge syndrome, ataxia-telangiectasia, and severe combined immunodeficiency disease, all of which produce severe immunologic defects. After diagnosis, the patient needs evaluation of adrenal, pituitary, thyroid, gonadal, pancreatic, and parathyroid function as well as careful follow-up. The disease is progressive, and most patients eventually develop endocrinopathy.
Treatment aims to control infection but isn’t always successful. Topical antifungal agents, such as clotrimazole, miconazole, and nystatin, are useful. They may be prescribed as mouthwashes or troches (lozenges) for 5 to 10 days.
Systemic infections may not be fatal, but they’re serious enough to warrant vigorous treatment. Ketoconazole and fluconazole have had some positive effect. Oral or I.M. iron replacement may also be necessary. Treatment may also include plastic surgery of the lesions, when possible, and counseling to help patients cope with their disfigurement.
Teach the patient about the disease’s progressive manifestations and emphasize the importance of seeing an endocrinologist for regular checkups.
Review other book chapters online related to Invasive candidiasis:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Professional Guide to Diseases (Eighth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2005 ISBN: 1-58255-370-X 
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